BioMed Central

World Journal of Surgical Oncology

ss

Open AcceCase reportPancreatic and multiorgan resection with inferior vena cava reconstruction for retroperitoneal leiomyosarcomaJohn A Stauffer1, G Peter Fakhre1, Marjorie K Dougherty2, Raouf E Nakhleh3, William J Maples4 and Justin H Nguyen*2

Address: 1Section of General Surgery, Mayo Clinic, Jacksonville, Florida, USA, 2Division of Transplant Surgery, Mayo Clinic, Jacksonville, Florida, USA, 3Department of Laboratory Medicine and Pathology, Mayo Clinic, Jacksonville, Florida, USA and 4Division of Hematology and Oncology (W.J.M.), Mayo Clinic, Jacksonville, Florida, USA

Email: John A Stauffer - stauffer.john@mayo.edu; G Peter Fakhre - fakhre.peter@mayo.edu; Marjorie K Dougherty - dougherty.marjorie@mayo.edu; Raouf E Nakhleh - not@valid.com; William J Maples - maples.william@mayo.edu; Justin H Nguyen* - nguyen.justin@mayo.edu

* Corresponding author

AbstractBackground: Inferior vena cava (IVC) leiomyosarcoma is a rare tumor of smooth muscle origin.It is often large by the time of diagnosis and may involve adjacent organs. A margin-free resectionmay be curative, but the resection must involve the tumor en bloc with the affected segment ofvena cava and locally involved organs. IVC resection often requires vascular reconstruction, whichcan be done with prosthetic graft.

Case presentation: We describe a 39-year-old man with an IVC leiomyosarcoma that involvedthe adrenal gland, distal pancreas, and blood supply to the spleen and left kidney. Tumor excisioninvolved en bloc resection of all involved organs with reimplantation of the right renal vein andreconstruction of the IVC with a polytetrafluoroethylene graft. The patient recovered withoutrenal insufficiency, graft infection, or other complications. Follow-up abdominal imaging at 1 yearshowed a patent IVC graft and no locally recurrent tumor. Prosthetic graft provides a sufficientdiameter and length for replacement conduit in extensive resection of IVC leiomyosarcoma.

Conclusion: To our knowledge, this is the first case of resection of an IVC sarcoma withprosthetic graft reconstruction in combination with pancreatic resection. Aggressive surgicalresection including vascular reconstruction is warranted for select IVC tumors to achieve apotentially curative outcome.

BackgroundInferior vena cava (IVC) leiomyosarcomas are rare malig-nancies; fewer than 300 have been reported in literature.This mesenchymal tumor is derived from medial smoothmuscle cells and most often originates from the IVC seg-ment between the hepatic veins and the renal veins [1]. Itis most commonly diagnosed in women in their sixth dec-

ade, and the tumors often reach large dimensions beforedetection because of an absence of symptoms [2-6]. Theyare slow-growing and potentially curable by complete andmargin-free resection but are well known to present diffi-culties in resection because of their location and involve-ment of surrounding organs and vascular structures.

Published: 6 January 2009

World Journal of Surgical Oncology 2009, 7:3 doi:10.1186/1477-7819-7-3

Received: 11 September 2008Accepted: 6 January 2009

This article is available from: http://www.wjso.com/content/7/1/3

© 2009 Stauffer et al; licensee BioMed Central Ltd. This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Page 1 of 5(page number not for citation purposes)

World Journal of Surgical Oncology 2009, 7:3 http://www.wjso.com/content/7/1/3

Locally involved organs are commonly the kidney, adre-nal gland, and liver [2,3,5-8]. Radical resection of thetumor en bloc with the affected segment of the vena cavahas been shown in multiple studies to be a feasible optionwith improved survival [1-3,5,6,9,10]. The pancreas is notoften involved with this retroperitoneal sarcoma, andpancreas resection may increase the risk of graft infection.Indeed, to our knowledge, a concomitant retroperitonealsarcoma resection and pancreas resection with IVC inter-position grafting have not been reported in literature. Wedescribe a patient with a leiomyosarcoma involving theleft kidney, left adrenal gland, and distal pancreas, whichrequired IVC resection followed by reconstruction withpolytetrafluoroethylene (PTFE).

Case presentationA previously healthy 39-year-old man presented to theemergency department with a 2-month history of inter-mittent dull abdominal ache with weight gain as well asintermittent right upper extremity numbness. Physicalexamination revealed a mildly obese abdomen with asubtle mass in the left upper quadrant. No lower extremityedema was noted. Abdominal magnetic resonance imag-ing revealed a 15 × 6 × 5-cm, well-circumscribed, preaorticretroperitoneal mass, which involved the IVC, causingmass effect on the surrounding organs (Figure 1a, b). Themass was believed to originate from the IVC but was with-out total IVC occlusion. Tumor involved the left renalartery, splenic artery, and distal pancreas. Hemoglobin,platelet, serum urea nitrogen, creatinine, liver functiontest, α-fetoprotein, carcinoembryonic antigen, and CA 19-

9 findings were all within normal limits. Cells obtained bycomputer tomographically guided needle biopsy stainedpositive for vimentin and desmin, confirming the masswas a high-grade retroperitoneal leiomyosarcoma. Furtherimaging revealed metastatic involvement of the fifth cer-vical vertebra and epidural membrane. Over the course ofthe next 8 months, the patient underwent a C5 corpec-tomy and fusion with removal of the epidural tumor forhis metastatic lesion and received 50.4 Gy intensity-mod-ulated radiation therapy to his abdomen, 43.2 Gy to hiscervical spine, and 4 cycles of ifosfamide and doxorubicinchemotherapy. Subsequent evaluation showed isolateddisease in the retroperitoneum, and the patient was con-sidered to be a candidate for resection with IVC recon-struction of his symptomatic primary tumor.

Surgical resection of the mass was performed through abilateral subcostal incision. Careful abdominal explora-tion confirmed the preoperative findings, and no othermetastases were present. The tumor appeared to arisefrom the IVC at the level of the left renal vein and extendintraluminally in the IVC up to the caudate lobe, involv-ing the left kidney, left adrenal gland, distal pancreas,splenic artery, and left renal artery. The tumor was mobi-lized en bloc with the left kidney, left adrenal gland, distalpancreas, and spleen. Proximal and distal control of theIVC was obtained, and the tumor was resected. The pan-creas was transected with a linear stapling device, themain pancreatic duct was identified and oversewn, and aclosed suction drain was placed at the transection site.Adequate margins were ensured by frozen section. IVC

a, Magnetic resonance imaging shows the tumor (large asterisk) lying to the left of the superior mesenteric artery (SMA), involving the distal pancreas anteriorly, the superior pole of the left kidney posteriorly, and extending into the inferior vena cava (IVC) (small asterisk)Figure 1a, Magnetic resonance imaging shows the tumor (large asterisk) lying to the left of the superior mesenteric artery (SMA), involving the distal pancreas anteriorly, the superior pole of the left kidney posteriorly, and extending into the inferior vena cava (IVC) (small asterisk). b, The tumor (large asterisk) involves and encases the left renal vein and extends into the IVC. SMA indicates superior mesenteric artery.

Page 2 of 5(page number not for citation purposes)

World Journal of Surgical Oncology 2009, 7:3 http://www.wjso.com/content/7/1/3

reconstruction was performed from the level of the cau-date lobe to the distal IVC in an end-to-end fashion witha 14-mm external ring-reinforced PTFE interposition graft(Figure 2). The graft was wrapped with omentum and iso-lated from the overlying viscera. The right renal vein wasreimplanted into the infrarenal IVC. Gross and his-topathologic examination revealed high-grade leiomyosa-rcoma originating from the IVC involving the adrenalgland and pancreas (Figure 3). After the operation, thepatient's renal function remained intact, and he was dis-charged from the hospital on postoperative day 17 onlow-dose oral anticoagulation for 3 months.

Postoperatively, the patient underwent adjuvant chemo-therapy with 4 cycles of docetaxel and gemcitabine, resec-tion of a metastatic left deltoid tumor mass, and 40.0 Gyof radiation therapy to his left upper extremity. Follow-upabdominal imaging at 1 year revealed no recurrentabdominal disease and a patent IVC graft (Figure 4).

DiscussionPrimary leiomyosarcoma of the IVC is a rare malignanttumor first described in 1891 by Perl at autopsy. The mostcommon presenting symptoms are abdominal pain, pal-pable abdominal mass, and lower limb edema [1]. How-ever, even with extensive caval involvement, severevenous obstructive symptoms are not often seen, proba-bly because of the development of extensive venous col-laterals, which maintain adequate flow around the level ofobstruction [2]. The segment of IVC between the renalveins and the hepatic veins (level II or middle segment) isthe most commonly affected location for all primary vas-cular tumors [3,5,6].

IVC leiomyosarcomas are relatively resistant to chemo-therapy and radiotherapy, and complete resection of thetumor is the only known method for a chance of cure. Theprognosis for leiomyosarcoma of the IVC treated medi-cally is poor, with an average survival of less than 3months [1]. However, in the past 2 decades, aggressivesurgical resection has yielded notable survival benefits,even for patients with metastatic disease. While data areconfined to a relatively small number of patients, 5-yearsurvival rates have been shown to be as high as 31% to53% [3,5-8,10] after complete resection of level II IVC lei-omyosarcoma.

Early diagnosis is rare, and the tumors often invade sur-rounding organs. The amount of vascular involvement bythe retroperitoneal tumor accounts for the high surgicalrisk and technical difficulties seen during attempts at com-plete resection. Accurate preoperative imaging to deter-mine the extent of the tumor is essential for adequateplanning, and magnetic resonance imaging is the pre-ferred modality.

Caval management after IVC resection is controversial.Options include primary repair, autologous patching,ligation, or reconstruction with prosthetic graft. Extensivevenous involvement and large tumor size often precludeshort segment resection with simple repair or patching.Ligation of the IVC is favored by some and has beenshown to be well tolerated and generally safe, especially inthose with preoperative IVC thrombosis [1,3]. However,there is a risk of late complications such as pain, swelling,and skin breakdown from severe lower extremity edema.Long-term anticoagulation may be necessary in thesepatients. Suprarenal IVC tumor involvement treated withIVC ligation can place a patient at serious risk for renalinsufficiency. Restoration of flow to the right renal vein byreimplantation (or pelvic kidney autotransplantation) ismandatory to maintain right kidney function, butoptional for the left renal vein because of the left kidney'sconsiderable collateral drainage through the adrenal, infe-rior phrenic, gonadal, and paravertebral vessels [11].

Because of the considerable size of these tumors at diag-nosis, wide retroperitoneal dissection is often necessaryfor complete tumor resection, disrupting the preexistingvenous channels. This dissection negates any collateralflow that achieved venous decompression preoperatively.Long segments of tumor involvement of the IVC necessi-tate ligation of a larger amount of lumbar veins that serveas collaterals. Kieffer et al [5] used a proximal pressurereading of 30 mm Hg or more in the IVC as an indicationfor caval reconstruction and found reconstruction to benecessary in most cases. PTFE is the most commonly usedprosthetic material and has been shown to be a suitablereplacement for the IVC with excellent long-term patency

The interposition polytetrafluoroethylene graft (asterisk) was anastomosed superiorly to the inferior vena cava (IVC) just below the liver, and inferior to the infrarenal IVC, the right renal vein (RRV) was reimplanted into the native IVCFigure 2The interposition polytetrafluoroethylene graft (asterisk) was anastomosed superiorly to the inferior vena cava (IVC) just below the liver, and inferior to the infrarenal IVC, the right renal vein (RRV) was reimplanted into the native IVC.

Page 3 of 5(page number not for citation purposes)

World Journal of Surgical Oncology 2009, 7:3 http://www.wjso.com/content/7/1/3

[5,6,8-10,12]. Infection and graft thrombosis are the 2major complications of this type of reconstruction, butboth are rare. Graft thrombosis may or may not have anyclinical importance, and methods used to decrease itsincidence include the use of ring-reinforced PTFE to pre-vent compression, short-term anticoagulation, and place-ment of an arteriovenous fistula to augment flow [5].

Although increasing the complexity of the operation,partial or total resection of locally involved organs isnecessary for complete tumor removal because progno-sis is highly dependent on a tumor-free margin. Patientswith inadequate resections are at high risk for localrecurrence, causing death from a retroperitoneal sar-coma [3]. Multivisceral resection, especially of entericorgans, may make a surgeon hesitant to place autoge-nous material for reconstruction. However, PTFE graft

infection after IVC replacement has been shown to be arare occurrence in several large series [5,6,8-10,12].Measures to decrease risk of graft contamination includeroutine perioperative intravenous antibiotics, antibioticirrigation of the abdomen, and coverage of the graft withomentum for graft isolation. However, to our knowl-edge, resection of the pancreas has not been reported incombination with IVC resection and reconstruction.Pancreatic fistula occurs in up to 23% to 26% of cases ofdistal pancreatectomy for malignancy [13,14]. Pancre-atic leak would have serious consequences in the face ofprosthetic vascular material in close proximity and couldresult in catastrophic graft infection. Measures to preventpancreatic contamination of the graft should be under-taken, including ensuring adequate distal pancreaticstump closure and providing sufficient closed suctiondrainage of the pancreatic bed.

a, Specimen contained tumor mass, left kidney, left adrenal gland, spleen, and distal pancreasFigure 3a, Specimen contained tumor mass, left kidney, left adrenal gland, spleen, and distal pancreas. b, Leiomyosar-coma is seen in the lumen of the vena cava. The vena cava wall is on the right. c, Tumor fills the bottom of the picture pushing into the adrenal gland seen at the top of the picture. d, Tumor has replaced a portion of the pancreas. A pancreatic islet com-plex is marked (hematoxylin and eosin, original magnification × 20).

Page 4 of 5(page number not for citation purposes)

World Journal of Surgical Oncology 2009, 7:3 http://www.wjso.com/content/7/1/3

ConclusionAlthough often not curative, aggressive surgical resectioncombined with chemoradiotherapy has been definitivelyshown to prolong survival in patients with IVC leiomy-osarcomas. Vascular reconstruction is often required, andprosthetic replacement of the IVC with PTFE has beenshown to be a safe option for retroperitoneal sarcomas.Graft-related complications are low but may be increasedby tumor involvement of the pancreas. However, pancre-atic involvement did not preclude resection in this case,giving the patient the survival benefit of a margin-free rad-ical en bloc resection.

AbbreviationsIVC: inferior vena cava; PTFE: polytetrafluoroethylene

ConsentWritten informed consent was obtained from the patientfor publication of this case report and any accompanyingimages. A copy of the written consent is available forreview by the Editor-in-Chief of this journal.

Competing interestsThe authors declare that they have no competing interests.

Authors' contributionsJAS participated in care of the patient and data collection,participated in study design, participated in literaturereview and manuscript drafting, participated in manu-

script writing and revision, and read and approved thefinal manuscript. GPF participated in care of the patientand data collection, participated in study design, and readand approved the final manuscript. MKD participated incare of the patient and data collection, participated instudy design, and read and approved the final manuscript.REN participated in data collection and study design andread and approved the final manuscript. WJM participatedin care of the patient and data collection, participated instudy design, and read and approved the final manuscript.JHN participated in care of the patient and data collection,participated in study design, participated in manuscriptwriting and revision, and read and approved the finalmanuscript. All authors read and approved the final man-uscript.

References1. Mingoli A, Feldhaus RJ, Cavallaro A, Stipa S: Leiomyosarcoma of

the inferior vena cava: analysis and search of world literatureon 141 patients and report of three new cases. J Vasc Surg1991, 14:688-699.

2. Dzsinich C, Gloviczki P, van Heerden JA, Nagorney DM, PairoleroPC, Johnson CM, Hallett JW Jr, Bower TC, Cherry KJ Jr: Primaryvenous leiomyosarcoma: a rare but lethal disease. J Vasc Surg1992, 15:595-603.

3. Hollenbeck ST, Grobmyer SR, Kent KC, Brennan MF: Surgicaltreatment and outcomes of patients with primary inferiorvena cava leiomyosarcoma. J Am Coll Surg 2003, 197:575-579.

4. Burke AP, Virmani R: Sarcomas of the great vessels: a clinico-pathologic study. Cancer 1993, 71:1761-1773.

5. Kieffer E, Alaoui M, Piette JC, Cacoub P, Chiche L: Leiomyosar-coma of the inferior vena cava: experience in 22 cases. AnnSurg 2006, 244:289-295.

6. Hines OJ, Nelson S, Quinones-Baldrich WJ, Eilber FR: Leiomyosar-coma of the inferior vena cava: prognosis and comparisonwith leiomyosarcoma of other anatomic sites. Cancer 1999,85:1077-1083.

7. Mingoli A, Sapienza P, Cavallaro A, Di Marzo L, Burchi C, GiannarelliD, Feldhaus RJ: The effect of extent of caval resection in thetreatment of inferior vena cava leiomyosarcoma. AnticancerRes 1997, 17:3877-3881.

8. Dew J, Hansen K, Hammon J, McCoy T, Levine EA, Shen P: Leiomy-osarcoma of the inferior vena cava: surgical managementand clinical results. Am Surg 2005, 71:497-501.

9. Schwarzbach MH, Hormann Y, Hinz U, Leowardi C, Bockler D, Mech-tersheimer G, Friess H, Buchler MW, Allenberg JR: Clinical resultsof surgery for retroperitoneal sarcoma with major bloodvessel involvement. J Vasc Surg 2006, 44:46-55.

10. Illuminati G, Calio' FG, D'Urso A, Giacobbi D, Papaspyropoulos V,Ceccanei G: Prosthetic replacement of the infrahepatic infe-rior vena cava for leiomyosarcoma. Arch Surg 2006,141:919-924.

11. Kraybill WG, Callery MP, Heiken JP, Flye MW: Radical resection oftumors of the inferior vena cava with vascular reconstruc-tion and kidney autotransplantation. Surgery 1997, 121:31-36.

12. Bower TC, Nagorney DM, Cherry KJ Jr, Toomey BJ, Hallett JW, Pan-neton JM, Gloviczki P: Replacement of the inferior vena cava formalignancy: an update. J Vasc Surg 2000, 31:270-281.

13. Pannegeon V, Pessaux P, Sauvanet A, Vullierme MP, Kianmanesh R,Belghiti J: Pancreatic fistula after distal pancreatectomy: pre-dictive risk factors and value of conservative treatment. ArchSurg 2006, 141:1071-1076.

14. Fahy BN, Frey CF, Ho HS, Beckett L, Bold RJ: Morbidity, mortality,and technical factors of distal pancreatectomy. Am J Surg2002, 183:237-241.

One-year follow-up magnetic resonance image shows patent polytetrafluoroethylene graft (asterisk) and no local tumor recurrenceFigure 4One-year follow-up magnetic resonance image shows patent polytetrafluoroethylene graft (asterisk) and no local tumor recurrence.

Page 5 of 5(page number not for citation purposes)